Familial renal amyloidosis is a form of amyloidosis primarily presenting in the kidney.It is associated most commonly with congenital mutations in the fibrinogen alpha chain and classified as a dysfibrinogenemia (see Hereditary Fibrinogen Aα-Chain Amyloidosis [en.wikipedia.org]

Leading experts from Latin America, Africa, Near and Middle East, Indian Subcontinent, Far East, Oceania and Australia present their expert insights into specific conditions, as well as progress and challenges in the development of the specialty. [books.google.com]

The types that present with renal disease are usually associated with mutations in the genes for either apolipoprotein AI, apolipoprotein AII, lysozyme or fibrinogen A alpha-chain. [ncbi.nlm.nih.gov]

• Hunting

  الصفحة 108 - Hunt SA, Baker DW, Chin MH, et al. [books.google.com]

• Scotoma

  Proteinuria and renal failure Liver and spleen - Organomegaly Single Canadian-Italian family Apolipoprotein AI deletion 70-72 Renal - Proteinuria and renal failure Liver and spleen - Organomegaly (usually well-preserved function) Retina - Central scotoma [emedicine.com]

• Central Scotoma

  scotoma Single family of British origin Apolipoprotein AI Leu75Pro Renal - Proteinuria and renal failure Liver and spleen - Organomegaly Italy – Variable penetrance Apolipoprotein AI Leu90Pro Cardiac - Heart failure Larynx - Dysphonia Skin – Infiltrated [emedicine.com]

• Histrionic Personality Disorder

  Personality Disorder Hot Flashes Hydrocephalus, Normal Pressure Hypercoagulable State Metabolic Syndrome Narcissistic Personality Disorder Nosocomial Infections Panic Disorder Peripheral Arterial Disease Pituitary Adenoma Plantar Fasciitis Pneumonia, [books.google.com]

• Breast Mass

  Mass Dyspepsia Fatigue Hypernatremia Hypocalcemia Jaundice, Neonatal [books.google.com]

• Primary Amenorrhea

  Amenorrhea, Secondary Back Pain, Acute Bleeding, Gastrointestinal Breast Mass Dyspepsia Fatigue Hypernatremia Hypocalcemia Jaundice, Neonatal [books.google.com]

• Central Scotoma

  scotoma Single family of British origin Apolipoprotein AI Leu75Pro Renal - Proteinuria and renal failure Liver and spleen - Organomegaly Italy – Variable penetrance Apolipoprotein AI Leu90Pro Cardiac - Heart failure Larynx - Dysphonia Skin – Infiltrated [emedicine.com]

Please consult your own licensed physician regarding diagnosis and treatment of any medical condition! Please see also our disclaimer. This site complies with the HONcode standard for health information: verify here. Database updated 2019-02-19. [diseasesdatabase.com]

الصفحة 24 - Third Report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III). ‏ الصفحة 134 - Sacks FM, Pfeffer MA, Moye LA et al. [books.google.com]

Prognosis of Amyloidosis Prognosis is largely dependent on the extent of organ damage when amyloidosis is first diagnosed. [myvmc.com]

Amyloidosis associated with multiple myeloma has the poorest prognosis, and death within one year is common. All forms of renal amyloidosis have a poor prognosis, but patients may remain stable and even improve with supportive therapy. [patient.info]

Prognosis Prognosis in amyloidosis is dependent primarily on the degree of cardiac involvement and, in AL amyloidosis, on circulating light-chain levels. [acc.org]

Prognosis - Familial Visceral Amyloidosis Not supplied. Treatment - Familial Visceral Amyloidosis Treatment is directed at eliminating the underlying cause and is mainly supportive. [checkorphan.org]

Dialysis improves the prognosis and survival, but morbidity and mortality are higher than in other populations given this treatment. [ojrd.biomedcentral.com]

Penetrance of this particular mutation is extremely low in most families obscuring the genetic etiology, but the renal histology is very characteristic showing substantial accumulation of amyloid within enlarged glomeruli, but none in blood vessels or [ncbi.nlm.nih.gov]

It usually manifests as nephrotic syndrome : fever abdominal pain hypertension proteinuria edema urinary symptoms when urinary tract is involved Etiology primary amyloidosis secondary amyloidosis: aging, chronic infection, chronic inflammatory diseases [radiopaedia.org]

Cornea The clinical appearance of corneal amyloidosis varies with the etiology but commonly features grayish subepithelial or intrastromal deposits in a filamentous or nodular pattern. Symptoms vary with the size, number and location of deposits. [healio.com]

Two patients underwent endomyocardial biopsies; one did not show amyloid and the other showed patchy amyloid deposits within the endocardium and interstitium in association with a dilated cardiomyopathy of undetermined etiology. [doi.org]

Noninvasive etiologic diagnosis of cardiac amyloidosis using 99m Tc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol. 2005;46:1076-84. Medline 66. [revespcardiol.org]

Epidemiological studies from Portugal and Sweden reveal that approximately one third of the patients display varying degrees of albuminuria and renal dysfunction. [ojrd.biomedcentral.com]

Epidemiology Frequency No systematic data address the frequency of FRA, but the condition is not as rare as previously thought. [emedicine.com]

Indeed, there are no epidemiological data to suggest that amyloid occurs as result of infection. More is known about how the antibody molecules are deposited in tissue and lead to the disease. [iupui.edu]

Epidemiology Amyloidosis is a rare disease. [patient.info]

Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices. [books.google.com]

Since then, an additional three mutations (encoding two protein variants) have been described in association with hereditary renal amyloidosis (see Pathophysiology). [emedicine.staging.medscape.com]

Therefore, we have two major objectives for our research: 1) to understand the pathophysiology of transthyretin amyloidosis so that means to treat or prevent the disease can be found, and 2) continue our study of this disease so that we may be able to [iupui.edu]

Islet amyloid and type 2 diabetes; from molecular misfolding to islet pathophysiology. Biochim Biophys Acta. 2001;1537:179-203. Sanchorawala V, Wright DG, Seldin DC, et al. [rarediseases.org]

Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. ‏ [books.google.com]

Prevention - Familial Visceral Amyloidosis Not supplied. Diagnosis - Familial Visceral Amyloidosis Not supplied. Prognosis - Familial Visceral Amyloidosis Not supplied. [checkorphan.org]

There are no known ways to prevent most forms of amyloidosis. [healthcommunities.com]

Is it possible to prevent amyloidosis? There is no prevention for amyloidosis. However, the secondary forms of amyloidosis can be prevented by treating the underlying diseases that are associated with inflammation. [rxlist.com]

These treatments can give your body time to gradually clear the deposits before they build up again, and can help prevent organ damage. In most cases, the treatment will involve having chemotherapy. [nhs.uk]